THE BASAL CELL NEVUS SYNDROME

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Abstract
1. Four cases of the basal cell nevus syndrome occurring within a single family are described.2. The patients show many of the characteristic roentgen findings of the syndrome including mandibular cysts, calcification of the falx cerebri, vertebral anomalies, rib anomalies, and brachymetacarpalism.3. Findings of the syndrome are described in a 3 year old boy (Case IV), the youngest reported patient with both recognizable bone and skin anomalies.4. Shortening of the distal phalanx of the thumb was present in 2 of the 4 patients. This finding has not previously been reported in association with the basal cell nevus syndrome.

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